Birth Defects Coding
and ICD-10-CM
Janet Cragan, MD, MPH
National Birth Defects Prevention Network (NBDPN)
18th Annual Meeting
October 19, 2015
National Center on Birth Defects and Developmental Disabilities
Division of Birth Defects and Developmental Disabilities
Chapter 17. Congenital Malformations,
Deformations and Chromosomal Abnormalities
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Q00-Q07
Q10-Q18
Q20-Q28
Q30-Q34
Q35-Q37
Q38-Q45
Q50-Q56
Q60-Q64
Q65-Q79
Congenital malformations of the nervous system
Congenital malformations of eye, ear, face, and neck
Congenital malformations of the circulatory system
Congenital malformations of the respiratory system
Cleft lip and cleft palate
Other congenital malformations of the digestive system
Congenital malformations of genital organs
Congenital malformations of the urinary system
Congenital malformations and deformations of the
musculoskeletal system
 Q80-Q89 Other congenital malformations
 Q90-Q99 Chromosomal abnormalities, not elsewhere classified
Improved Defect Coding in ICD-10-CM

Increased specificity
 ICD-9-CM: 750.3 – Tracheoesophageal fistula, esophageal atresia and
stenosis
 ICD-10-CM:
•
•
•
•
•

Q39.0 – Atresia of esophagus without fistula
Q39.1 – Atresia of esophagus with tracheo-esophageal fistula
Q39.2 – Congenital tracheo-esophageal fistula without atresia
Q39.3 – Congenital stenosis and stricture of esophagus
Q39.4 – Esophageal web
Laterality incorporated into defect codes
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Q65.00 – Congenital dislocation of unspecified hip, unilateral
Q65.01 – Congenital dislocation of right hip, unilateral
Q65.02 – Congenital dislocation of left hip, unilateral
Q65.1 – Congenital dislocation of hip, bilateral
Improved Defect Coding in ICD-10-CM
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Gastroschisis and omphalocele
 ICD-9-CM: Both included in 756.79 – Anomalies of abdominal wall
 ICD-10-CM:
• Q79.2 – Exomphalos, Omphalocele
• Q79.3 – Gastroschisis
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Arnold-Chiari syndrome
 ICD-9-CM: Included in 741.0 - Spina bifida with hydrocephalus
 ICD-10-CM:
•
•
•
•
Q07.00 – Arnold-Chiari syndrome without spina bifida or hydrocephalus
Q07.01 – Arnold-Chiari syndrome with spina bifida
Q07.02 – Arnold-Chiari syndrome with hydrocephalus
Q07.03 – Arnold-Chiari syndrome with spina bifida and hydrocephalus
Changes to Defect Coding in ICD-10-CM

Persistent fetal circulation
 ICD-9-CM: Included under 747.8 – Other specified anomalies of circulatory system
 ICD-10-CM: Included under P29 – Cardiovascular disorders originating in the perinatal
period
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Neurofibromatosis
 ICD-9-CM: Included under 237 – Neoplasm of uncertain behavior of endocrine glands
and nervous system
 ICD-10-CM: Included under Q85 – Phakomatoses, not elsewhere classified

Fetal alcohol syndrome
 ICD-9-CM: Included under 760.7 – Noxious influences affecting fetus or newborn via
placenta or breast milk
 In ICD-10-CM: Included under Q86 – Congenital malformation syndromes due to
known exogenous causes, not elsewhere classified
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Epispadias
 ICD-9-CM: Included under 752 – Congenital anomalies of genital organs
 ICD-10-CM: Included under Q64 – Other congenital malformations of urinary system
Changes to Defect Coding in ICD-10-CM
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Megalencephaly vs. macrocephaly
 ICD-9-CM: Macroencephaly and megalencephaly included under 742.4 – Other
specified anomalies of brain
• There is no code for macrocephaly
 ICD-10-CM: Megalencephaly included under Q04 – Other congenital
malformations of brain
• Macrocephaly included under Q75 – Other congenital malformations of skull
and face bones
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Conditions not listed in ICD-9-CM but added to ICD-10-CM
 Congenital subglottic stenosis (Q31.1)
 Congenital laryngomalacia (Q31.5)
 Congenital tracheomalacia (Q32.0)
• These conditions can be congenital, but often are related to prematurity or
prolonged intubation. Many programs do not include them as congenital
malformations.
Changes to Defect Coding in ICD-10-CM
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Cleft lip and/or cleft palate
 ICD-9-CM
•
•
•
•
Unilateral vs. bilateral for both
Complete vs. incomplete for both
Includes cleft lip and/or cleft palate, unspecified
Does not distinguish hard vs. soft palate
 ICD-10-CM
• Unilateral, bilateral, median for cleft lip
• Hard vs. soft cleft palate
• Includes unspecified cleft palate (with or without cleft lip), but not unspecified
cleft lip alone (included under unilateral cleft lip)
• Does not distinguish complete vs. incomplete for either
Problematic Coding in ICD-10-CM
Heart Valves
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ICD-9-CM
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Pulmonary valve: atresia, stenosis, other, unspecified
Tricuspid valve: atresia and stenosis, Ebstein anomaly
Aortic valve: stenosis, insufficiency
Mitral valve: stenosis, insufficiency
ICD-10-CM
 Pulmonary valve: atresia, stenosis, insufficiency, other
 Tricuspid valve: stenosis (includes atresia), Ebstein anomaly, other,
unspecified
 Aortic valve: stenosis (includes atresia), insufficiency
 Mitral valve: stenosis (includes atresia), insufficiency
 Aortic and mitral valves: other, unspecified
Problematic Coding in ICD-10-CM
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Atrial septal defect (ASD) and patent foramen ovale (PFO)
 ICD-9-CM: 745.5 – Ostium secundum type ASD (includes ASD and PFO)
 ICD-10-CM: Q21.1 – Atrial septal defect (includes ASD and PFO)
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Interrupted aortic arch (IAA)
 ICD-9-CM: 747.11 – Interruption of aortic arch
 In ICD-10-CM: IAA is not mentioned anywhere
• Could be coded under Q25.2 – Atresia of aorta or Q25.4 – Other congenital
malformations of aorta
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Clinical genetic syndromes
 Many more specific syndromes mentioned
 Some interspersed throughout defect codes
 Conditions lumped together into single codes based on a single clinical
feature (facial appearance, short stature, limb involvement, etc.)
NBDPN Annual Report
Probable Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Holoprosencephaly
742.2
Q04.2
ICD-9-CM includes other
reduction defects
Decrease
Cloacal exstrophy
751.5
Q64.12
ICD-9-CM includes other
anomalies of intestine
Decrease
Congenital posterior
urethral valves
753.6
Q64.2
ICD-9-CM includes other atresia
and stenosis of bladder neck
Decrease
Craniosynostosis
756.0
Q75.0
ICD-9-CM includes other
anomalies of skull and face
bones
Decrease
Interrupted aortic
arch (IAA)
747.11
Q25.2,
Q25.4
ICD-10-CM does not specify a
code for IAA
Increase
Clubfoot
754.51,
754.70
Q66.0,
Q66.89
ICD-10-CM includes other
congenital deformities of feet
Increase
NBDPN Annual Report
No Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Anophthalmia/Microphthalmia
743.0, 743.1
Q11.0-Q11.2
--------------------
No change
Congenital cataract
743.30-743.34
Q12.0
--------------------
No change
Anotia/Microtia
744.01, 744.23
Q16.0, Q17.2
--------------------
No change
Atrioventricular septal defect/
Endocardial cushion defect
745.60, 745.61,
745.69
Q21.2
--------------------
No change
Ebstein anomaly
746.2
Q22.5
--------------------
No change
Hypoplastic left heart syndrome
746.7
Q23.4
--------------------
No change
746.01, 746.02
Q22.0, Q22.1
--------------------
No change
Tricuspid valve atresia and
stenosis
746.1
Q22.4
--------------------
No change
Choanal atresia
748.0
Q30.0
--------------------
No change
Biliary atresia
751.61
Q44.2-Q44.3
--------------------
No change
Pulmonary valve atresia and
stenosis
NBDPN Annual Report
No Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Cleft lip with cleft palate
749.2
Q37.0-Q37.9
--------------------
No change
Cleft lip alone
749.1
Q36.0-Q36.9
--------------------
No change
Cleft palate alone
749.0
Q35.1-Q35.9
--------------------
No change
Esophageal atresia/
Tracheoesophageal fistula
750.3
Q39.0-Q39.4
--------------------
No change
Bladder exstrophy
753.5
Q64.10, Q64.19
--------------------
No change
Hypospadias
752.61
Q54.0-Q54.9
excluding Q54.4
--------------------
No change
Renal agenesis/hypoplasia
753.0
Q60.0-Q60.6
--------------------
No change
Diaphragmatic hernia
756.6
Q79.0, Q79.1
--------------------
No change
Limb deficiencies/
Reduction defects of
lower and unspecified
limb
755.3-755.4
Q72.0-Q72.9,
Q73.0-Q73.8
--------------------
No change
NBDPN Annual Report
No Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Deletion 22q11.2
758.32
Q93.81
--------------------
No change
Trisomy 13
758.1
Q91.4-Q91.7
--------------------
No change
Trisomy 18
758.2
Q91.0-Q91.3
--------------------
No change
Trisomy 21/Down syndrome
758.0
Q90.0-Q90.9
--------------------
No change
NBDPN Annual Report
Possible Small Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Anencephalus
740.0-740.1
Q00.0-Q00.1
ICD-10-CM includes acephaly
Small
increase
Spina bifida without
anencephalus
741.0, 741.9
without
740.0-740.1
Q05.0-Q05.9,
Q07.01, Q07.03
without Q00.0-Q00.1
ICD-9-CM may include
Arnold Chiari w/o spina
bifida
Small
decrease
Encephalocele
742.0
Q01.0-Q01.9
ICD-10-CM includes ArnoldChiari type III
Small
increase
Aortic valve stenosis
746.3
Q23.0
ICD-10-CM excludes aortic
valve stenosis in HLHS
Small
decrease
Atrial septal defect
745.5
Q21.1
ICD-10-CM specifies more
individual types
Small
increase
Coarctation of
the aorta
747.1
Q25.1
ICD-9-CM includes
hypoplasia of aorta and IAA;
ICD-10-CM codes hypoplasia
elsewhere, no code for IAA
Small
decrease
Common truncus/
Truncus arteriosus
745.0
Q20.0
ICD-10-CM excludes aortic
septal defect
Small
decrease
NBDPN Annual Report
Possible Small Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Double outlet right
ventricle
745.11
Q20.1
ICD-9-CM includes
dextrotransposition of the aorta
Small
decrease
Single ventricle
745.3
Q20.4
ICD-10-CM specifies double inlet
ventricle
Small
increase
Tetralogy of Fallot
745.2
Q21.3
ICD-9-CM includes Pentalogy of
Fallot
Small
decrease
Total anomalous
pulmonary venous
connection
747.41
Q26.2
ICD-10-CM has a separate code for
unspecified anomalous pulmonary
venous connection
Small
decrease
Transposition of the
great arteries
745.10,
745.12,
745.19
Q20.3, Q20.5
ICD-10-CM specifies ventricular
inversion and levotransposition,
includes dextrotransposition of the
aorta
Small
increase
745.4
Q21.0
ICD-9-CM includes Eisenmenger’s
defect
Small
decrease
Ventricular septal
defect
Comments
Effect on
Prevalence
NBDPN Annual Report
Possible Small Change in Defect Prevalence
Defect
ICD-9-CM
Codes
ICD-10-CM
Codes
Comments
Effect on
Prevalence
Limb deficiencies/
Reduction defects of
upper limb
755.2
Q71.0-Q71.9
ICD-10-CM includes lobster-claw
hand
Small increase
Rectal and large
intestinal
atresia/stenosis
751.2
Q42.0-Q42.9
ICD-9-CM includes obstruction of
large intestine
Small decrease
Small intestinal
atresia/stenosis
751.1
Q41.0-Q41.9
ICD-9-CM includes obstruction of
small intestine, NOS
Small decrease
Gastroschisis
756.73
Q79.3
Previous ICD-9-CM code 756.7
included omphalocele
Decrease
or no change
Omphalocele
756.72
Q79.2
Previous ICD-9-CM code 756.7
included gastroschisis
Decrease
or no change
Turner syndrome
758.6
Q96.0-Q96.9
ICD-10-CM codes pure gonadal
dysgenesis elsewhere
Small decrease
or no change
ICD-9-CM to ICD-10-CM Code Translation Tools

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Developed for birth defect programs that primarily use ICD-9-CM
for defect coding
Began by identifying all valid alternative ICD-10-CM codes in the
general equivalence mappings (GEMs) developed by NCHS
 Made modifications appropriate for birth defects surveillance needs
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For each code in ICD-9-CM, the tool provides possible alternative
codes in ICD-10-CM
 Many ICD-9-CM codes have only one corresponding code in ICD-10-CM
 When more than one possible alternative is available, programs can select
the one that most closely matches the defect
 If there is not enough information about the defect to select among
multiple alternatives, a default code is designated as the preferred code
 The tool contains every code in ICD-9-CM, but not necessarily every code
in ICD-10-CM
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An ICD-10-CM to ICD-9-CM code translation tool is also available
from the NBDPN website
CDC Expanded Code for Birth Defects
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Intended for use by programs that currently use the CDC/BPA
code or a modification of it
Analogous to ICD-10-CM as the CDC/BPA code is to ICD-9-CM
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Expand the range of numbers in each ICD-10-CM code
Maintain collapsibility to ICD-10-CM
Increase specificity, laterality, degree of severity, location of defect, etc.
Add specific conditions not coded in ICD-10-CM (e.g., syndromes)
Update terminology/medical knowledge
Resolve issues that were problematic in the CDC/BPA code
Ensure that all sections have codes for “Other” and “Unspecified” defects
CDC Expanded Code for Birth Defects

Development began in the early 1990’s
 Identified defects frequently coded in the “Other” or “Other specified”
categories in MACDP in order to give them individual codes
 Identified defects recommended by MACDP staff and expert clinicians that
are not coded in CDC/BPA
 Identified codes and conditions that had been added or modified in the
CDC/BPA code by other programs that use it
 Reviewed the literature to identify conditions or variants not currently
coded in CDC/BPA

Next will share with state programs that do their own coding
 Assess utility and obtain feedback, corrections, advice
 Still need to harmonize the expansion with defect codes in BD-STEPS

MACDP plans to begin using the expanded code for births in
January 2016
 Continue to use the CDC/BPA code for births through December 2015
ICD-10-CM Tools and Resources
 NBDPN Website Page on ICD-10-CM and Birth Defects:
http://www.nbdpn.org/icd9_icd10_code_translation.php
• Code Translations from ICD-9-CM to ICD-10-CM and from ICD-10-CM to
ICD-9-CM for Birth Defects Surveillance (Excel files)
• ICD-10-CM Implementation Plan Template
• Slides from three webinars on “Transition to Use of ICD-10-CM Coding for
Birth Defects”
 NBDPN Coding Tools Work Group Message Board (Members Only
Section):
http://www.viethconsulting.com/members/forum/board_list.php
• NBDPN members can post questions about ICD-10-CM, share experiences
and tips, discuss common concerns, etc.
ICD-10-CM Tools and Resources
 National Center for Health Statistics:
http://www.cdc.gov/nchs/icd/icd10cm.htm
• 2016 release of ICD-10-CM Code, Guidelines, and General Equivalence
Mapping files
 Centers for Medicare & Medicaid Services:
https://www.cms.gov/Medicare/Coding/ICD10/2016-ICD-10-PCSand-GEMs.html
• 2016 release of ICD-10-PCS Code, Guidelines, Addendum, and General
Equivalence Mapping files
 CDC Website on Public Health Transition to ICD-10-CM/PCS:
http://www.cdc.gov/nchs/icd/icd10cm_pcs.htm
• Transition Planning, Trainings, Resources, FAQs
 American Health Information Management Association (AHIMA):
http://www.ahima.org/topics/icd10
• ICD-10- Implementation Toolkit, Preparation Checklist
Acknowledgements
Tricia Bhatti
Cynthia Moore
Sonja Rasmussen
Claude Stoll
Donna Pickett, NCHS
For more information, contact CDC
1-800-CDC-INFO (232-4636)
TTY: 1-888-232-6348 www.cdc.gov
The findings and conclusions in this presentation are those of the authors and do not necessarily
represent the official position of the Centers for Disease Control and Prevention.
National Center on Birth Defects and Developmental Disabilities
Division of Birth Defects and Developmental Disabilities